Rett Syndrome and Diet

Diet is a very important factor for proper brain development both during fetal growth and during postnatal life. In neurodevelopmental disorders such as Rett syndrome, a proper diet is essential so that the child gets adequate nutrition to help effectively manage symptoms.

Dietary inadequacy in Rett syndrome

Rett syndrome is a disorder characterized by impaired brain development. It is caused by mutations in the MECP2 gene that contains the information necessary for the production of a protein called MeCP2. This protein acts as a switch that turns many genes on and off including those in nerve cells.

Impaired brain development also results in several other issues that can cause nutritional deficiencies. For example, Rett syndrome patients usually have upper gastrointestinal complications, gastroparesis (inability of the stomach to empty food into the small intestine), gastroesophageal reflux disease (GERD; heartburn due to stomach acid flowing back into the esophagus), and dysphagia (difficulty in swallowing), among others. These symptoms drastically affect nutritional intake and proper weight gain.

Recent studies in animal models have shown that Rett syndrome patients are predisposed to excess calorie intake and, consequently, obesity. Although the exact underlying mechanism is still unclear, it has been postulated that the parts of the brain responsible for fat metabolism and feeding behavior are likely affected by MECP2 gene mutations.

Nutrition goals for management of Rett syndrome symptoms

For the effective management of Rett syndrome symptoms, a proper diet plan is essential. The diet plan should:

  • be well-balanced with adequate energy, protein, calcium, and vitamin D
  • be able to prevent weight loss and help maintain proper body mass index (BMI)
  • take into account the most appropriate feeding methods for the individual
  • consider pre-existing symptoms such as low bone mineral density, GERD, dysphagia, and dehydration

Feeding strategies for Rett syndrome patients

Taking into account the above factors, a proper diet can be formulated for the individual in consultation with the clinician (usually a pediatrician). Following a few simple steps can ensure that Rett syndrome patients are able to take in the required amounts of nutrition to maintain their overall health. It is important to ensure a calm feeding environment and proper body positioning that helps in easy swallowing and minimal distraction.

Rett syndrome patients often have difficulty communicating their food needs. Parents and caregivers also face challenges in making their child understand they need to eat. Using visual cues or actions could be useful to encourage the child to eat.

The texture of the food should be such that it is easy to swallow without much effort. The food may be mashed, chopped, or pureed as needed. Liquids are best served thickened to prevent choking.

Fiber and fluid intake may be increased if symptoms of constipation are seen. Parents and caregivers should consult a healthcare provider if the patient needs medication to control constipation. Nutrition supplements may be used for increased protein intake if recommended by the clinician.

Some patients with Rett syndrome also experience seizures. It has been demonstrated that the ketogenic diet can help reduce seizures. The ketogenic diet is a high-fat diet with low carbohydrates and limited proteins. It can compensate for the energy requirement of the brain as the liver converts fats into ketone bodies in a process known as ketogenesis. Ketone bodies are an important energy source for the brain in the absence of carbohydrates. However, the ketogenic diet presents its own risks such as kidney and liver problems, obesity, nutritional deficiency, and mood swings so it is best implemented under the supervision of a clinician or a registered dietitian.

Parents and caregivers must ensure that diet plans and feeding strategies are followed exactly as prescribed to ensure sufficient nutrition and proper development. In case of unavailability, parents and caregivers must provide for alternate arrangements to ensure that the daily nutrition intake of the patient is not affected.


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